Hematology Practice Questions

Q: Which of the following is the most common manifestation of hemophilia?

Hemarthrosis. **Explanation:** **Hemophilia** (Factor VIII or IX deficiency) is a disorder of secondary hemostasis. Unlike primary hemostatic defects (platelet disorders), which present with superficial bleeding, secondary hemostatic defects typically present with **deep-seated bleeding**. **Why Hemarthrosis is Correct:** Hemarthrosis (bleeding into joint spaces) is the hallmark and most common clinical manifestation of hemophilia, occurring in approximately 75–90% of patients. It most frequently affects the **hinge joints**, with the **knee** being the most common site, followed by the elbow and ankle. Recurrent hemarthrosis leads to "hemophilic arthropathy," characterized by synovial hypertrophy and joint destruction. **Analysis of Incorrect Options:** * **A & C (Hemoptysis and Hematemesis):** These represent internal mucosal or visceral bleeding. While they can occur in severe hemophilia following trauma or underlying pathology (like an ulcer), they are rare and not characteristic primary presentations. * **D (Mucosal bleeding):** This includes epistaxis, gum bleeding, and petechiae. These are classic features of **primary hemostasis defects** (e.g., Von Willebrand Disease or Thrombocytopenia). In hemophilia, mucosal bleeding is uncommon unless there is significant trauma. **High-Yield Clinical Pearls for NEET-PG:** * **Most common joint involved:** Knee > Elbow > Ankle > Hip > Shoulder. * **First sign of hemophilia in infants:** Prolonged bleeding after circumcision or cephalhematoma. * **Muscle Hematomas:** The second most common site of bleeding; the **Iliopsoas muscle** is a high-yield site to remember (can mimic appendicitis). * **Lab Findings:** Prolonged aPTT, normal PT, and normal Bleeding Time (BT). * **Life-threatening complication:** Intracranial hemorrhage is the leading cause of death in hemophiliacs.

Q: A 10-year-old boy complains of fatigue and abdominal pain after playing football. He also complains of painful swelling of digits in the past. Ultrasound abdomen shows a small spleen. Which of the following is the most likely diagnosis?

Sickle cell anemia. ### Explanation The clinical presentation is classic for **Sickle Cell Anemia (SCA)**. The key diagnostic clues are: 1. **Dactylitis (Hand-Foot Syndrome):** The "painful swelling of digits" in early childhood is often the first manifestation of SCA, caused by infarcts in the small bones of the hands and feet. 2. **Vaso-occlusive Crisis (VOC):** Fatigue and abdominal pain after physical exertion (football) suggest a crisis triggered by dehydration or hypoxia. Abdominal pain in SCA can result from mesenteric ischemia or splenic sequestration. 3. **Autosplenectomy:** The ultrasound finding of a **"small spleen"** is a pathognomonic late feature of SCA. Repeated splenic infarctions lead to fibrosis and shrinkage of the spleen (autosplenectomy), usually by age 8–10. --- ### Why the other options are incorrect: * **Iron Deficiency Anemia:** While it causes fatigue, it does not present with dactylitis, acute abdominal pain, or splenic atrophy. * **Pancreatitis:** Though it causes abdominal pain, it does not explain the history of painful digits or a shrunken spleen. * **Megaloblastic Anemia:** Presents with fatigue and glossitis, but not with vaso-occlusive phenomena or autosplenectomy. --- ### NEET-PG High-Yield Pearls: * **Splenomegaly in SCA:** The spleen is initially enlarged in early childhood (due to congestion) before undergoing **autosplenectomy**. If a teenager with SCA has a palpable spleen, suspect **HbSC disease** or **S-β thalassemia**. * **Howell-Jolly Bodies:** These nuclear remnants on a peripheral smear indicate functional asplenia/autosplenectomy. * **Most common organism for Osteomyelitis in SCA:** *Salmonella* (though *S. aureus* remains common overall). * **Most common cause of death in adults:** Acute Chest Syndrome. * **Management:** Hydroxyurea is used to increase **HbF** levels, which inhibits sickling.

Q: What is the most frequent cause of neonatal thrombocytopenic purpura?

Infection. **Explanation:** **1. Why Infection is the Correct Answer:** In neonatal medicine, **infection (sepsis)** is the most common cause of thrombocytopenia. It occurs in approximately 20–50% of neonates admitted to the NICU. The mechanism is multifactorial: it involves increased platelet consumption due to disseminated intravascular coagulation (DIC), direct bone marrow suppression by toxins, and immune-mediated destruction. Both bacterial (Gram-negative sepsis) and viral (TORCH infections like CMV) etiologies are significant contributors. **2. Analysis of Incorrect Options:** * **Drug Idiosyncrasy (B):** While certain maternal drugs (e.g., thiazides, hydralazine) can cause neonatal thrombocytopenia, this is rare compared to the high incidence of neonatal sepsis. * **Large Haemangiomas (C):** This refers to **Kasabach-Merritt Syndrome**, where platelets are sequestered within a vascular tumor. While it causes severe thrombocytopenia, it is a rare clinical entity. * **Erythroblastosis (D):** In Rh-incompatibility (Erythroblastosis Fetalis), thrombocytopenia can occur due to hypersplenism or marrow replacement by erythroid hyperplasia, but it is far less frequent than infection-induced causes. **3. NEET-PG High-Yield Pearls:** * **Definition:** Neonatal thrombocytopenia is defined as a platelet count ** 72 hours):** Almost always due to **Sepsis** or **Necrotizing Enterocolitis (NEC)**. * **Immune causes:** If the neonate is otherwise healthy but has severe thrombocytopenia, consider **Neonatal Alloimmune Thrombocytopenia (NAIT)**—the most common cause of *isolated* severe thrombocytopenia in an otherwise well term infant.

Q: A child presents with sudden onset of red spots on the body, preceded by a viral infection 1-4 weeks prior. Investigations show Hb 12 gm%, WBC 11,000, and platelet count of 50,000. Which of the following is not useful for the management of this condition?

Platelet transfusion. **Explanation:** The clinical presentation of sudden onset petechiae/purpura following a viral prodrome, with isolated thrombocytopenia (Platelets 50,000/µL) and normal hemoglobin/WBC counts, is classic for **Immune Thrombocytopenic Purpura (ITP)**. **Why Platelet Transfusion is NOT useful (Correct Option):** In ITP, the underlying pathology is the production of autoantibodies (IgG) against platelet surface antigens. These antibody-coated platelets are rapidly destroyed by splenic macrophages. If exogenous platelets are transfused, they are immediately targeted and cleared by the same circulating antibodies. Therefore, platelet transfusion is ineffective and reserved only for life-threatening emergencies (e.g., intracranial hemorrhage). **Why other options are used in management:** * **Corticosteroids (Option D):** The first-line treatment. They decrease antibody production and reduce the clearance of platelets by the reticuloendothelial system. * **Intravenous Immunoglobulin (IVIG) (Option B):** Used for rapid elevation of platelet counts. It works by "clogging" the Fc receptors on splenic macrophages, preventing them from destroying platelets. * **Intravenous anti-D therapy (Option C):** An alternative for Rh-positive patients. It causes mild hemolysis; the spleen becomes busy clearing antibody-coated RBCs, thereby sparing the platelets. **Clinical Pearls for NEET-PG:** * **Bone Marrow Aspiration:** Not mandatory in typical cases but shows **increased megakaryocytes** (compensatory). * **Most common cause of isolated thrombocytopenia** in children. * **Prognosis:** 80% of childhood ITP cases are self-limiting and resolve spontaneously within 6 months. * **Management Threshold:** Treatment is usually initiated if the platelet count is **<20,000/µL** or if there is significant mucosal bleeding.

Q: Anemia, splenomegaly, and structural defects are typically seen in which of the following conditions?

Thalassemia major. **Explanation:** **Thalassemia Major** (also known as Cooley’s Anemia) is the correct answer because it represents the most severe form of beta-thalassemia, characterized by a near-total lack of beta-globin chain synthesis. This leads to a triad of clinical features: 1. **Severe Anemia:** Ineffective erythropoiesis and hemolysis of defective red blood cells lead to profound microcytic hypochromic anemia, usually manifesting within the first 6–9 months of life. 2. **Splenomegaly:** Massive enlargement occurs due to both extramedullary hematopoiesis and the sequestration of damaged RBCs. 3. **Structural Defects:** To compensate for chronic anemia, the bone marrow undergoes massive expansion. This results in characteristic skeletal changes such as **"Chipmunk facies"** (prominent maxilla, malocclusion of teeth) and a **"Hair-on-end"** appearance on skull X-rays. **Incorrect Options:** * **Thalassemia Minor:** Usually asymptomatic or presents with mild anemia. Patients do not exhibit significant splenomegaly or skeletal deformities. * **Mediterranean Anemia:** This is a historical synonym for Thalassemia. However, in the context of this question, "Thalassemia Major" is the specific clinical entity that defines the severe phenotype described. * **Polycythemia:** This condition involves an *increase* in red blood cell mass, which is the physiological opposite of anemia. **NEET-PG High-Yield Pearls:** * **Target Cells:** Commonly seen on peripheral smears of Thalassemia patients. * **Mentzer Index:** (MCV/RBC count) 13 suggests Iron Deficiency Anemia. * **Hb Electrophoresis:** In Thalassemia Major, there is a marked increase in **HbF** and absence/reduction of HbA. * **Iron Overload:** The primary cause of mortality in these patients (due to repeated transfusions), leading to restrictive cardiomyopathy and endocrinopathies.

Q: The parents of an 8-year-old boy complained of frequent severe bleeding on minor trauma. The patient could be suffering from which of the following conditions?

Hemophilia A. **Explanation:** The clinical presentation of frequent, severe bleeding following minor trauma in a young male child is a classic indicator of a **coagulation factor deficiency**, most commonly **Hemophilia A**. **1. Why Hemophilia A is correct:** Hemophilia A is an X-linked recessive disorder caused by a deficiency of **Factor VIII**. It typically manifests in males. The hallmark of the disease is "deep-seated" bleeding. While severe cases present in infancy (e.g., post-circumcision), moderate to mild cases often present in childhood with excessive bruising, hematomas, or **hemarthrosis** (bleeding into joints) following minor physical activity or trauma. **2. Why the other options are incorrect:** * **Anemia:** This refers to a decrease in red blood cells or hemoglobin. While it causes pallor, fatigue, and tachycardia, it does not inherently cause a bleeding diathesis. * **Attention Deficit Hyperactive Disorder (ADHD):** This is a neurobehavioral disorder. While a hyperactive child might sustain more injuries, ADHD itself does not cause a physiological bleeding tendency. * **Disseminated Intravascular Coagulation (DIC):** While DIC causes severe bleeding, it is an **acute, life-threatening consumptive coagulopathy** secondary to systemic illness (sepsis, trauma, malignancy). It would not present as a chronic history of "frequent" bleeding in an otherwise stable 8-year-old. **High-Yield Clinical Pearls for NEET-PG:** * **Inheritance:** Hemophilia A and B are X-linked recessive; Hemophilia C (Factor XI deficiency) is Autosomal Recessive. * **Lab Findings:** Characterized by **Prolonged aPTT** with a **Normal PT and Bleeding Time**. * **Mixing Study:** aPTT will **correct** when the patient's plasma is mixed with normal plasma (distinguishes deficiency from inhibitors). * **Treatment:** Recombinant Factor VIII concentrate is the treatment of choice. Desmopressin (DDAVP) can be used in mild cases.

Q: A 9-year-old child presents with continuous bleeding after tonsillectomy. Bleeding time and PTT are prolonged, while platelet count and PT are normal. What is the most likely diagnosis?

Von Willebrand disease. ### Explanation The clinical presentation of post-surgical bleeding combined with a specific laboratory profile points toward **Von Willebrand Disease (vWD)**, the most common inherited bleeding disorder. **Why Von Willebrand Disease is Correct:** Von Willebrand Factor (vWF) has two primary roles: 1. **Platelet Adhesion:** It acts as a bridge between platelets and the subendothelium. A deficiency leads to a defect in the platelet plug formation, resulting in a **prolonged Bleeding Time (BT)**. 2. **Carrier for Factor VIII:** vWF stabilizes Factor VIII in circulation. Low levels of vWF lead to a secondary decrease in Factor VIII, which causes a **prolonged Partial Thromboplastin Time (PTT)**. Since vWD does not affect the extrinsic pathway or platelet production, the **PT and Platelet count remain normal**. **Why Other Options are Incorrect:** * **Vitamin K Deficiency:** Affects Factors II, VII, IX, and X. This would primarily prolong the **PT** (due to Factor VII's short half-life) and PTT, but BT would be normal. * **Immune Thrombocytopenic Purpura (ITP):** Characterized by isolated **thrombocytopenia** (low platelet count). While BT would be prolonged, the PTT would be normal. * **Hemophilia A:** An isolated deficiency of Factor VIII. It causes a prolonged PTT, but the **BT is normal** because platelet-vWF interaction is unaffected. **High-Yield Clinical Pearls for NEET-PG:** * **Inheritance:** Most common type (Type 1) is Autosomal Dominant. * **Screening Tests:** Prolonged BT + Prolonged PTT + Normal Platelet Count. * **Confirmatory Test:** Ristocetin Cofactor Assay (measures vWF activity). * **Treatment of Choice:** Desmopressin (DDAVP), which releases stored vWF from Weibel-Palade bodies. For severe cases, use vWF-containing concentrates.

Question 1

Which of the following is the most common manifestation of hemophilia?

Accepted Answer

Hemarthrosis

Answer

Hemoptysis

Answer

Hematemesis

Answer

Mucosal bleeding

Question 2

A 10-year-old boy complains of fatigue and abdominal pain after playing football. He also complains of painful swelling of digits in the past. Ultrasound abdomen shows a small spleen. Which of the following is the most likely diagnosis?

Accepted Answer

Sickle cell anemia

Answer

Iron deficiency anemia

Answer

Pancreatitis

Answer

Megaloblastic anemia

Question 3

What is the most frequent cause of neonatal thrombocytopenic purpura?

Accepted Answer

Infection

Answer

Drug idiosyncrasy

Answer

Large haemangiomas

Answer

Erythroblastosis

Question 4

A child presents with sudden onset of red spots on the body, preceded by a viral infection 1-4 weeks prior. Investigations show Hb 12 gm%, WBC 11,000, and platelet count of 50,000. Which of the following is not useful for the management of this condition?

Accepted Answer

Platelet transfusion

Answer

Intravenous Immunoglobulin

Answer

Intravenous anti-D therapy

Answer

Corticosteroids

Question 5

Anemia, splenomegaly, and structural defects are typically seen in which of the following conditions?

Accepted Answer

Thalassemia major

Answer

Thalassemia minor

Answer

Mediterranean anemia

Answer

Polycythemia

Question 6

The parents of an 8-year-old boy complained of frequent severe bleeding on minor trauma. The patient could be suffering from which of the following conditions?

Accepted Answer

Hemophilia A

Answer

Anemia

Answer

Attention Deficit Hyperactive Disorder

Answer

Disseminated intravascular coagulation

Question 7

A 5-year-old boy is being evaluated for recurrent epistaxis and other abnormal bleeding episodes, including excessive bleeding from the umbilical cord. Laboratory studies reveal the following: decreased hemoglobin (with microcytic hypochromic red cell indices), normal platelet count, markedly prolonged prothrombin time (PT) and partial thromboplastin time (PTT), and unmeasurable thrombin time (TT). Platelet aggregation studies reveal a normal platelet response to ristocetin, but with other substances (including collagen, ADP, and epinephrine), this patient's platelets exhibit a primary wave defect. Based on these findings, what is the most likely diagnosis?

Accepted Answer

Afibrinogenemia

Answer

Bernard-Soulier syndrome

Answer

Glanzmann's thrombasthenia

Answer

Wiskott-Aldrich syndrome

Question 8

Hemolytic crisis in hereditary spherocytosis is most commonly precipitated by which of the following?

Accepted Answer

Parvovirus B19 infection

Answer

Infectious mononucleosis

Answer

Human T-cell leukemia virus

Answer

Cytomegalovirus

Question 9

A 9-year-old child presents with continuous bleeding after tonsillectomy. Bleeding time and PTT are prolonged, while platelet count and PT are normal. What is the most likely diagnosis?

Accepted Answer

Von Willebrand disease

Answer

Vitamin K deficiency

Answer

Immune thrombocytopenic purpura

Answer

Hemophilia A

Question 10

What is the first biochemical change noted after iron therapy is initiated?

Accepted Answer

Decreased transferrin saturation

Answer

Reticulocytosis

Answer

Increase in serum ferritin levels

Answer

Replenishment of iron stores

Hematology — MCQs

Hematology — MCQs

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